Establishment of a pilot pediatric registry for chronic vasculitis is both essential and feasible: a Childhood Arthritis and Rheumatology Alliance (CARRA) survey.

OBJECTIVE

To identify the need for, and feasibility of, establishing a web-based USA/Canadian registry of children with chronic systemic vasculitis--an otherwise insufficiently studied population.

METHODS

Physician members of the Childhood Arthritis and Rheumatology Research Alliance (CARRA; n = 126) were invited to complete 2 surveys exploring vasculitis-related experience, beliefs about childhood versus adult vasculitis, and commitment to contribute patients to a prospective registry. Diagnoses included Wegener's granulomatosis (WG), childhood polyarteritis nodosa, microscopic polyangiitis (MPA), Takayasu's arteritis, primary angiitis of the central nervous system (PACNS), vasculitis, and unclassified vasculitis.

RESULTS

One or both surveys were completed by 102 (81%) physicians. Almost half of first-survey respondents had been in practice for > 15 years. Collective estimated lifetime experience was >1500 patients (WG and unclassified vasculitis were the most common diagnoses). Three hundred seventeen children with vasculitis were seen in the year preceding the survey, with most physicians seeing only 2-5 patients. The majority of respondents believed that childhood vasculitis differed from adult disease, particularly with respect to classification criteria and disease activity markers. Fifty-nine members committed to contribute 2 years' data (approximately 120 patients) to a pilot registry limited to time of diagnosis, focusing on WG, MPA, Churg-Strauss syndrome, PACNS, and unclassified vasculitis.

CONCLUSION

We obtained overwhelming consensus from an experienced body of pediatric rheumatologists on the need to study childhood-onset vasculitis independently from adult disease, together with commitment from sufficient members to prospectively contribute 2 years' data to a limited pilot registry to answer some basic questions about presenting and diagnostic features and initial treatment practices at disease onset.